Analysis Tools
behavior/neurological
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• mice take longer to traverse the elevated beam at 7 months but not at earlier time points, with mice showing footslips when crossing the narrowest beam
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• mice show a motor deficit on the accelerating rotarod at 7 months of age and not earlier, which progressively worsens with age, indicating that disease onset occurs rapidly around 7 months of age
• mice take longer to traverse the elevated beam at 7 months but not at earlier time points, with mice showing footslips when crossing the narrowest beam
• while swimming performance shows no age-dependent differences, mutants produce a small but significant increase in hindlimb kicks at 7 months on the third day
• however, mice do not show gait abnormalities before or at the onset of motor coordination deficits and have normal stride length and width
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nervous system
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• Purkinje cell degeneration occurs at 2 years of age, about 1.5 years after the onset of motor deficits, with 2 year old mice having about 22% fewer Purkinje cells than wild-type mice
• Purkinje cell density is reduced in both anterior and posterior lobules of the vermis
• however, no differences in striatal neuron density is seen at 7 months of age
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• average molecular layer thickness is reduced about 15% at 2 years of age compared to at 7 months of age
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| spinocerebellar ataxia type 6 | DOID:0050956 |
OMIM:183086 |
J:239981 | |
